Importance: Patients with sickle cell disease may develop various macular vascular abnormalities that have not been described previously and can be seen using optical coherence tomographic angiography.
Observations: Ten eyes from 5 consecutive patients (3 men and 2 women) with sickle cell disease (4 patients with hemoglobin SS disease and 1 patient with hemoglobin SC disease) were included. The mean age was 37.6 years. Five of 10 eyes (50%) had retinal thinning that was identified using spectral-domain optical coherence tomography. Each of these eyes had corresponding loss of vascular density in the superficial or deep retinal plexus (or both).
Conclusions and relevance: Optical coherence tomographic angiography provides a novel way to view the retinal vasculature, including the superficial and deep capillary plexus, and may provide a sensitive method for identifying macular vascular abnormalities in patients with sickle cell disease.